Background and objectives : The diagnosis of Pierre Robin sequence (PRS) has always been controversial. A very large database (3791 cleft patients) with 237 cases of PRS, divided into the Siebold–Robin sequence (SRS) and Fairbairn–Robin triad (FRT) were evaluated. The diagnostic criteria, oligohydramnios theory, mandibular catch-up-growth, midfacial hyperplasia, and peri-surgical management are presented.

Methods : A comprehensive literature survey was done and the results of the clinic’s database analyzed for a comparison. The main question is: Is PRS a glossoptosis in the presence of a mandibular micrognathia with a compromized airway OR a cleft palate with mandibular micrognathia and glossoptosis? Are these two different entities with an identical airway problem? 237 PRS patients were analyzed and divided into the SRS (micrognathia and glossoptosis) and the FRT (cleft palate, plus SRS features). A comprehensive literature survey was conducted and with the clinic’s database compared.

Results : The 237 cases were divided into syndromic and non-syndromic groups. The SRS were not associated with any syndrome, however, 46 (20.7%) FRT cases were associated with syndromes. One hundred and forty-four mothers could supply their pregnancy history. An oligohydramnios was verified in 22 (15.8%) FRT and 3 (60.0%) SRS cases. Catch-up-growth could be verified in 45 (35.4%) of 127 evaluated cases. Normal midfacial growth occurred in 72.6% and 5.3% presented with a midfacial hypoplasia. Management of PRS is multifaceted, and is determined by: airway compromise; feeding problems; sequence of palatal reconstruction (FRT) (sP 7 months; hP 18 months), and orthognathic deformities.

Conclusion : This PRS database appears to be the largest in the world with 237 patients. The incidence is 5.9% (222 FRT of 3791 cleft cases). No specific reason of the development of a micrognathia in utero, the cornerstone theory for oligohydramnios, and the subsequent mandibular catch-up growth could be determined. The subcategories of SRS/FRT have a wider spectrum of diversity than assumed.